Extranasopharyngeal angiofibroma in children: A case report.

BACKGROUND: Sporadic cases of extranasopharyngeal angiofibroma in children, especially preschool children, have been reported in the literature. CASE
SUMMARY: We present a case of extranasopharyngeal angiofibroma in a 4-year-old boy. The presenting symptoms, imaging findings, treatment, histological appearance, and follow-up data are described in detail. For this patient, we performed embolization on two occasions, and then, resected the tumor completely. During the treatment, the patient developed a soft-palate perforation due to aseptic necrosis. However, the healing ability was good, and the perforation healed spontaneously. We additionally reviewed all pediatric cases of extranasopharyngeal angiofibroma published up to 30 June 2020 in the PubMed, Baidu Scholar, Scopus, and Web of Science databases. We identified 45 pediatric patients [average (10.98 ± 4.86), boys 39 (86.7%)]. The highest proportion of cases occurred in adolescence [22 (48.9%)]. The top three sites of occurrence of extranasopharyngeal angiofibroma in children were the maxillary sinus, nasal septum, and inferior turbinate.
CONCLUSION: Extranasopharyngeal angiofibromas can occur throughout childhood, and predominantly present with nasal obstruction and spontaneous rhinorrhagia. ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.

Core Tip: Extranasopharyngeal angiofibromas can occur throughout childhood, but predominantly occur during adolescence. They present with similar symptoms. In our case, the clinical presentation was unusual due to the patient’s young age, the large tumor size, and the requirement of two embolization treatments. Soft palate perforation developed as a complication, but healed spontaneously during follow-up. Additionally, we performed a literature review to summarize the clinical characteristics of this rare tumor.

INTRODUCTION

Angiofibromas are nasopharyngeal tumors that predominantly occur in adolescent boys. These tumors almost always originate in the region of the sphenopalatine foramen and enlarge to fill the postnasal space[1,2]. Angiofibromas that do not originate in this region are rare, and referred to as extranasopharyngeal angiofibromas[3]. Sporadic cases of extranasopharyngeal angiofibromas have been reported in the literature[2]. These types of angiofibromas most commonly originate from the maxillary sinus. Here, we describe a rare case of an extranasopharyngeal angiofibroma arising from the inferior turbinate and lateral wall of the nasopharynx in a child. The clinical presentation was unusual due to the patient’s young age, the large tumor size, and the requirement of two embolization treatments. Soft palate perforation developed as a complication, but healed spontaneously during follow-up. Additionally, we performed a literature review to summarize the clinical characteristics of this rare tumor.

CASE PRESENTATION

Chief complaints

A 10-d history of epistaxis and nasal obstruction in the left nasal cavity.

History of present illness

A 4-year-old male reported to the ENT outpatient department of our institution with the chief complaints of epistaxis and nasal obstruction in the left nasal cavity for 10 d. The child had a sudden left nasal hemorrhage during sleep 10 days before, which was too large to stop by itself. He went to a local hospital for emergency treatment and stopped bleeding after filling the left nasal cavity with an inflated sponge. The next day, the child removed the expansive sponge by himself without obvious bleeding.Since then, the left nasal obstruction was persistent, accompanied by white purulent nasal discharge, which was difficult to blow out due to a large amount. Blood was sometimes seen in the nose, along with sleep snoring, accompanied by open mouth breathing and suffocation during sleep. Therefore, the patient was admitted to our hospital.

History of past illness

The child was previously healthy.

Physical examination

A pinkish lesion was seen in the total nasal passages of Bilateral nasal cavity

Imaging examinations

A Computed Tomography Scan of the paranasal sinuses was done which showed bilateral posterior nasal passage obstructiona, a 2.5 cm × 2.7 cm size Soft tissue density shadow in the nasopharyngeal,and there was no bony erosion.Enhanced Magnetic Resonance Imaging (MRI) of paranasal sinus presented abnormal signal of nasopharynx with obvious enhancement, consideration of adolescent nasopharyngeal fibroangioma (Figure 1).

Figure 1

Enhanced magnetic resonance imaging showing an abnormal signal in the nasopharynx with obvious enhancement.

FINAL DIAGNOSIS

Nasopharyngealangiofibroma.

TREATMENT

After two embolization treatments, we use a plasma knife to remove the tumor (Figure 2).

Figure 2

Before and after embolization. A: Pre-embolization angiography reveals that the tumor (arrow) is supplied by the left internal maxillary artery; B: After the second embolization, the arteriovenous fistula in the tumor in the nasopharynx has almost disappeared.

OUTCOME AND FOLLOW-UP

The tumor tissues were completely removed. A follow-up nasal endoscopy at 3 mo after the endoscopic excision showed smooth mucosa at the posterior end of the left inferior turbinate and on the lateral wall of the nasopharynx, with no obvious new lesions.

DISCUSSION

Nasopharyngeal angiofibroma is a benign tumor of the nasopharynx. These tumors mostly occur in adolescent boys, and appear to be more common in the Middle East and Indian subcontinent[4]. Although the etiology and pathogenesis of nasopharyngeal angiofibroma are not yet clear, juvenile nasopharyngeal angiofibroma is believed to be caused by insufficient estrogen or relatively excessive androgen, which leads to hyperplasia of the vascular and fibrous tissues[5].

Due to the locally invasive nature of this tumor, it can widely involve the nasopharynx, paranasal sinuses, the orbital, pterygopalatine, and inferior temporal fossae, and even invade the skull base and cavernous sinus. Angiofibroma of extranasopharyngeal origin is rare. Extranasopharyngeal tumors most commonly originate in the maxillary sinus, but have also been reported to originate in the ethmoid sinus, sphenoid sinus, frontal sinus[7], middle turbinate[8], inferior turbinate[9], septum[10], cheek[11], and conjunctiva[6]. We searched the PubMed, Baidu Scholar, Scopus, and Web of Science databases for the search term “extranasopharyngeal angiofibroma.” A review of the retrieved literature revealed that a total of 45 cases, including ours, of this tumor have been reported in children[1,2,12,13]. We have also formulated a table that was first compiled by Ali and Jones in 1982 and updated it with recent cases of extranasopharyngeal angiofibroma in children (age < 18 years), including the present case (Table 1)[1]. The main features of these cases have been summarized in Table 2.

Table 1

Characteristics of extranasopharyngeal angiofibromas in children

No	Ref.	Site of origin	Age	Sex
1	Munson[21], 1941	Maxillary sinus	15 years	M
2	Radcliffe[22], 1951	Ethmoid sinus	16 years	M
3	Alajmo and Fini-Storchi[26], 1962	Maxillary sinus	9 years	M
4	Whitlock et al[23], 1961	Cheek	16 years	M
5	Alajmo and Fini-Storchi[26], 1962	Maxillary sinus	6 years	M
6	Hora and Brown[26], 1962	Maxillary sinus	13 years	M
7	Furstenborg and Boles[26], 1963	Ethmoid sinus	1 months	M
8	Minicone[26], 1964	Conjunctiva	17 years	M
9	Ogura[26], 1965	Maxillary sinus	16 years	M
10	Chaikovskii[26], 1967	External nose	14 years	F
11	Szczepanski et al[24], 1967	Ethmoid sinus	13 years	F
12	Manigla[26], 1969	Maxillary sinus	15 years	M
13	Pathak[26], 1970	Maxillary sinus	18 years	M
14	Beeden and Alexander[26], 1971	Oropharynx and hypopharynx	1 years	M
15	Charkabti[26], 1973	Maxillary sinus	17 years	M
16	Rye[26],1973	Maxillary sinus	17 years	M
17	Stewart and O’Brien[26], 1973	Molar and retromolar	10 years	M
18	Ramajaneyulu[26], 1974	Maxillary sinus	17 years	M
19	Yamagiwa[26], 1974	Sphenoid sinus	14 years	M
20	Isherwood et al[25], 1975	Pterygomaxillary fissure, infratemporal region	13 years	M
21	Krutchkoff[30], 1977	Maxillary sinus	12 years	M
22	Reddy[26], 1979	Molar and retromolar area	14 years	F
23	Obiako et al[31], 1983	Roof of nasal cavity	12 years	M
24	Hiraide and Matsubara[27], 1984	Nasal septum	13 years	M
25	Sarpa and Novelly[28], 1989	Nasal septum	9 years	M
26	Kitano et al[32], 1992	Maxillary sinus	13 years	M
27	Manjalay et al[33], 1992	Maxillary sinus	Newborn	M
28	Gaffney et al[18], 1997	Inferior turbinate	9 years	M
29	Schick et al[6], 1997	Lacrimal sac	15 months	M
30	Schick et al[6], 1997	Paranasal sinus	9 years	M
31	Schick et al[6], 1997	Sphenoid sinus	6 years	M
32	Huang et al[8], 2000	Middle turbinate	14 years	M
33	Handa et al[12], 2001	Nasal septum	8 years	M
34	Panesar et al[2], 2003	Maxillary sinus	1 years	M
35	Gupta et al[34], 2006	Infratemporal region	13 years	M
36	Castillo et al[29], 2006	Nasal septum	9 years	M
37	Ifeacho and Caulfield[35], 2011	Middle turbinate	14 years	M
38	Singhal et al[17], 2014	Nasal septum	12 years	M
39	Ganguly et al[16], 2017	Nasal septum	7 years	M
40	Singh et al[17], 2018	Nasal septum	9 years	F
41	Kim et al[20], 2019	Inferior turbinate	9 years	M
42	Kim et al[20], 2019	Inferior turbinate	16 years	M
43	Kim et al[20], 2019	Inferior turbinate	8 years	F
44	Kim et al[20], 2019	Inferior turbinate	12 years	F
45	Our case	Inferior turbinate and lateral wall of nasopharynx	4 years	M

Table 2

Reported cases of extranasopharyngeal angiofibroma in children (n = 45)

Variable	N (%)
Gender
Male	39 (86.7%)
Female	6 (13.3%)
Age at the time of surgery
≤ 6 years	8 (17.8%)
7-12 years	15 (33.3%)
13-18 years	22 (48.9%)
Site
Maxillary sinus	15 (33.3%)
Ethmoid sinus	3 (6.7%)
Sphenoid sinus	2 (4.4%)
Nasal septum	7 (15.6%)
Inferior turbinate	6 (13.3%)
Middle turbinate	2 (4.4%)
Roof of nasal cavity	1 (2.2%)
Lacrimal sac	1 (2.2%)
Cheek	1 (2.2%)
Conjunctiva	1 (2.2%)
External nose	1 (2.2%)
Molar and retromolar area	2 (4.4%)
Oropharynx and hypopharynx	1 (2.2%)
Infratemporal region	2 (4.4%)

Of the 45 patients with extranasopharyngeal angiofibroma, 39 (86.7%) were boys, and 6 (13.3%) were girls, yielding a male-to-female ratio of 13:2. The highest proportion of cases occurred in adolescence (48.9%), which is consistent with the reported prevalence of the disease in adolescents[2]. The second most affected age group was children aged 7-12 years, who accounted for 35.6% of cases. Infants accounted for the smallest proportion of cases (15.5%). Thus, we found that extranasopharyngeal angiofibromas can occur throughout childhood, with non-adolescent children accounting for half of the total number of cases. The top three sites of occurrence of extranasopharyngeal angiofibroma in children were the maxillary sinus, nasal septum, and inferior turbinate, which accounted for 31.1%, 20%, and 13.3% of cases, respectively. Other sites were less frequently involved, and included the ethmoid sinus (6.7%), sphenoid sinus (4.4%), and middle turbinate (4.4%).

The main clinical manifestations of extranasopharyngeal angiofibroma were nasal obstruction (80%) and spontaneous rhinorrhagia (60%). Other manifestations included secondary headache; sinusitis symptoms; tumor invasion of the pharyngeal opening of the eustachian tube, leading to conductive hearing loss; and tumor enlargement causing cheek swelling[14]. In our patient, the main symptoms were nasal obstruction and nosebleed, which is consistent with the reported findings.

Preoperative examinations for extranasopharyngeal angiofibroma include nasal endoscopy, CT, MRI, and angiography, which are required to fully evaluate the tumor extent, blood supply, and main blood vessels. Prior to the clinical diagnosis, intraoperative hemorrhage can be avoided by performing preoperative angiography and feeding-vessel embolization[15]. However, none of the children in the previous cases underwent preoperative embolization. This shows that extranasopharyngeal angiofibroma is less vascular, and thus, the chances of massive hemorrhage are low. For our patient, we chose to perform a second embolization 28 d after the first embolization, and then, remove the tumor. This is because the tumor had an abundant blood supply, was large, and its boundaries could not be clearly determined. Moreover, angiography showed early arteriovenous enhancement and abundant arteriovenous malformation, so we could not rule out the possibility of arteriovenous fistula (Figure 3A). Therefore, considering the high risk of intraoperative bleeding and the difficulty in achieving complete tumor resection, we planned to perform a second embolization after tumor necrosis and shrinkage had set in (Figure 3B).

Figure 3

Transformation of soft palate perforation. A: After the first embolization, an irregular perforation is seen in the left soft palate; B: At 3 mo after the endoscopic excision, the necrotic area in the left soft palate has completely healed.

After the first embolization, the patient developed soft palate necrosis as a complication, which has not been previously reported (Figure 4A). The patient experienced reflux of food through the perforation in the soft palate and into the nasal cavity. As the perforation gradually narrowed, this symptom lessened and then disappeared. It is possible that the arteries supplying the soft palate were simultaneously embolized during the first embolization of the pterygoid segment of the internal maxillary artery, resulting in ischemic necrosis of the soft palate. However, because the perforation was caused by aseptic necrosis, it healed well, and had completely closed by the time of the reexamination 3 mo after the operation (Figure 4B).

Figure 4

The posterior end of the inferior turbinate and lateral wall of the nasopharynx after resection of the tumor mass.

The main treatment for extranasopharyngeal angiofibroma is surgical resection, which is usually performed via the transnasal endoscopic, lateral rhinotomy incision, or transoral approaches, depending on the site and size of the tumor. We reviewed the surgical procedures performed for extrapharyngeal angiofibromas in children over the last 30 years, and found that tumors in the septum, inferior turbinate, and middle turbinate were mostly commonly removed using nasal endoscopic resection (e.g., Ganguly et al[16] and Singh et al[17]). Gaffney et al[18] and Huang et al[8] performed lateral rhinotomy to remove tumors originating from the inferior and middle turbinates, respectively. Handa et al[12] used an external approach incision in the left alar crease to resect a nasal septum tumor, which was found to be firmly adhered to the nasal septum at the junction of the quadrangular cartilage and the bony septum. Endoscopic and KTP laser-assisted surgery has also been used[19]. Panesar et al[2] used the endoscopic approach for the first time to resect a tumor located in the maxillary sinus; however, their patient developed tumor recurrence after the operation, and another midfacial degloving procedure was used for the complete removal of the recurrent tumor. In the present study, we used a nasal endoscopic approach. During the surgery, the tumor was completely excised along with the tissues in a 0.5-cm margin around the tumor to minimize intraoperative hemorrhage. We used a plasma knife to remove the tumor, which helped to clear the operative field and reduced the probability of intraoperative bleeding and complications. There was little intraoperative bleeding in our patient, and nasal packing was not performed after tumor removal, which helped to minimize postoperative pain.

CONCLUSION

Extranasopharyngeal angiofibromas can occur throughout childhood, but predominantly occur during adolescence. They present with similar symptoms such as nasal obstruction and spontaneous rhinorrhagia. When post-embolization complications occur, like in our case, they can be a challenge to manage and treat. However, aseptic necrosis due to embolism is associated with good healing ability and spontaneous repair.

ACKNOWLEDGEMENTS

The authors express their appreciation for Dr. Liu Yang’s assistance with the pathology micrographs.